The silent killer

On 28 May 1998, a day much like any other, Ashley Jolly was still in bed when his mother left the house for work. But he was working hard for his GCSEs, so she left him alone. After all, his younger brother Philip, who shared his bedroom, hadn’t got up either.

Later that morning, Anne Jolly phoned home. Nobody answered. That’s strange, she thought. John was away on business, but where were her sons? A bit later, she tried again. After a long wait, Philip answered. He’d obviously just got out of bed. Anne asked him, “Isn’t Ashley awake?” Philip went back to his room to have a look, then returned to the phone: “I can’t wake him,” he said. “He won’t wake up, Mum.’

Ashley was born in 1982. When the midwife put him into the cot beside Anne’s bed, she looked at him and thought, ‘My God, from now on this little person has to be looked after.’

He was always active. In 1998, aged 16, he played football twice a week, cycled 56 miles from London to Brighton, and ran for his athletics team. At school, computing was his best subject. He’d set up his own website – quite an achievement back then. Against a blue background, this showed a “Welcome” sign with flashing lights, a whirring globe, and Homer Simpson bobbing up and down inside a vast beer glass. The centre of the page announced the time of day, and greeted visitors warmly, particularly if they visited more than once: “Wow, I must be doing a darn good job!”

On the phone from work, Anne told Philip to fetch a neighbour, Sharon. Meanwhile she called for an ambulance and hurried home. When she got there, Sharon looked grave. Anne rushed into the bedroom and looked at her older qson. He looked peaceful, as if asleep, but showed no sign of life. The paramedics confirmed that Ashley was dead.

How could that be? How could somebody so young die suddenly in his sleep? Ashley had always been so healthy. His immune system seemed impenetrable. He’d even won awards for taking no time away from school.

As it turns out, somebody young and healthy dies mysteriously every day. In fact, more often than that: eight die each week of instantaneous heart failure, classified as Sudden Adult Death Syndrome (Sads). Ciara Agnew, 14, died coming home on the school bus. Tom Baldwin, 15, died at a friend’s house. Jamie Coffey, 17, was having a kickabout after dinner when he collapsed and died. Sarah Dudhill, 19, was found dead in bed by her boyfriend. Robert Manning, 16, died playing rugby with his father and brothers… The list goes on and on. Each one of these individuals was previously regarded as healthy. So who’s to say this couldn’t happen to someone in your family?

The average age for sudden cardiac deaths is 17, but Sads does not only kill teenagers. One of the most recent – unconfirmed – cases was Dan Frank, who collapsed and died without warning in October, in the newsroom of the Catholic Herald, where he worked. He was 24. Still older victims include Tony Hughes, 35, who also died at work, and father-of-three Richard Abbott, 34.

If these people had been murdered, the killer would by a considerable margin have outdone even the prolific Harold Shipman. Each death would command the front pages until the killer was caught. But they’re not murders, and they’re actually so common that they’re rarely reported in the news beyond the local paper. Consequently, families like the Jollys usually have little idea that their private loss fits into a national pattern.

Until relatively recently, the same could have been said for cot death, which kills barely half as many people – fewer than 250 infants each year. As awareness of cot death increased – notably thanks to the campaigning efforts of Anne Diamond – the incidence fell dramatically. If awareness of Sads reached the same levels, hundreds of people could be saved each year. And that might include you, or someone you love.

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Ashley’s death was a mystery. The police were called at once. John Jolly, summoned home from work, found an ambulance and police cars outside. Anne tried to explain, and John said he wanted to see Ashley. But the police held him back. Nobody was allowed into the bedroom till an inspector had been to take a look. Not even Philip, still wearing nothing but boxer shorts.

Eventually, Ashley was enclosed in a body bag and taken away. But the family would have to wait for a post mortem to find out how he had died.

“It sounds so dramatic when you try to explain it,” Anne says, sitting on the sofa at home in Brentwood. “But when something like this happens, you feel like your whole life has ended. You don’t think you’re going to survive. It’s physical pain, as well as emotional pain: your stomach actually hurts.”

Before I came to see Anne, I expected our conversation to be extremely painful. I was ready for Anne to break down in tears. That’s not what happened. Considering what she’s been through, Anne’s face is remarkably unlined – a clue, perhaps, that this is a woman who keeps the more operatic emotions under check. Only after we’ve been talking for an hour or so, when she recollects that sensation of physical pain, does she blink rapidly – the first, easily missed, evidence of strong emotion.

No bereavement is easy, but the loss of a child is hardest of all. Statistics show that if you experience the death of a child, the odds that your marriage will break up are doubled. What happened to the Jollys was arguably worse than most such bereavements, because in cases of chronic illness families can prepare for a child’s passing. Even a fatal accident can be easier, because that gives parents someone else to blame – and without that, research suggests, they blame themselves.

One of Anne’s strategies for making sense of Ashley’s death was to write poetry. “Did I dream I had a son, I hugged for 16 years,/ Along with the responsibility, hard work and the tears./ Did I dream I lost him one night, as he lay in bed asleep,/ Did he really die so easily and leave us all to weep?”

She became convinced that her son was still with her; that he sent his love by dropping white feathers. “They fell on my lap on the anniversary of Ashley’s death.”

But her immediate response was to puzzle over her son’s death. Ashley wore a brace for his teeth. Anne wondered if something had come loose and he’d choked on it. She asked herself what he’d eaten, that last day. A lot of bacon, she remembered. Could that have done it? Countless other possibilities suggested themselves, not a few of them ridiculous. But when something doesn’t make sense it’s difficult to be logical.

Eventually, the official verdict came in: Ashley had suffered a fatal asthma attack.

The family accepted what they were told, because the people who investigate such things are experts. But something didn’t seem right. Ashley had never had asthma. And his peaceful appearance in death gave no sign of disturbance. He was a big boy, and Anne – a light sleeper – often heard him moving about in bed. Surely she’d have heard if he had an asthma attack. Philip would have heard it too. It didn’t make sense.

She asked to see a copy of the post mortem. She knew it would be upsetting, but how could she be more upset than she already was? She needed to find out what was what.

“A lot of people might think, ‘Well, he’s dead and that’s it,’ but with someone you have loved and nurtured for 16 years, you don’t just say, ‘Oh well, it’s just one of those things.’ Not knowing what has happened to a loved one can be dreadful. It became all consuming, overwhelming.”

Her surviving son, Philip, became severely introverted. He lived in his room and Anne practically had to post his dinner under the door. He went through an angry phase, getting in fights at school. John, her husband, struggled to cope with what had happened. “He didn’t want to speak about it. I could see it in his face, and he wouldn’t answer when I brought the subject up.” So she locked herself away upstairs in Ashley’s old computer room. She started writing to the teaching hospitals in London, asking about asthma and respiratory problems. Eventually she got a letter from Guy’s Hospital saying the problem was more likely to have been Ashley’s heart. Thinking she might have solved the mystery, Anne had her son’s heart sent to a specialist.

But they could find nothing wrong. It was perfectly normal.

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At the top of the human heart sits a group of blood vessels shaped like a crown, or corona. The coronary arteries supply blood from the heart to the heart itself: the fuel that powers the body’s engine. Most heart-related death involves some kind of malfunction here. Typically, the coronary arteries become furred with cholesterol, severely restricting blood flow. Not all such “attacks” are fatal, but when they are, the cause of death is easy to identify at post mortem.

That kind of heart problem, relatively common in older people, is rarely seen in young adults. Here, a likelier cause of death is some kind of “arrhythmia”, or disturbed heartbeat. This can arise because the heart muscle is exceptionally enlarged, or because of weaknesses in the right ventricle. In both cases, again, the cause of death can usually be determined at post mortem.

An entirely different set of potentially fatal problems, only recently identified, stem from the heart’s electrical circuitry. The muscle of the heart comprises millions of cells. Each cell has pores allowing the passage in and out of chemicals that provide an electrical signal, which in turn stimulates the heart to beat, and thus pump blood. A disordered electrical signal can only show up while the person is alive; in death, the heart appears entirely normal.

Altogether, one in every 20 cases of sudden cardiac death, or about 500 every year in the UK, shows no cause. This “invisible” killer can strike suddenly. Symptoms include breathlessness and fainting – but in one-third of cases the first symptom is sudden death. This can be provoked by exercise and stress, the deep relaxation of sleep, or shock. Even the sudden noise of a doorbell or mobile phone has been known to trigger a fatal arrhythmia.

Up to ten years ago, if a young person died suddenly and nothing showed up at post mortem, it was put down as a “natural” event, and families were told not to worry. Even specialists had no idea what was going on, says Dr Sian Hughes, a pathologist specializing in heart problems at University College London. In her windowless office, she rises from her chair to pull a massive volume from her shelves, Muir’s Textbook of Pathology. It’s the 12th Edition, published in 1985; the cover is held together with sticky tape. “Let’s have a look,” she says, opening the index, then turning to the section on hearts. “There’s nothing. These problems were not even known about then.”

Sads is still not fully understood, but we do know that the old advice – not to worry, carry on as before – was wrong. Sudden Arrythmic Death Syndrome (as doctors more correctly term it) is usually inherited. The whole family may have the condition. If individual members aren’t checked, a second – or third, or fourth – shocking bereavement may occur. In many families, that has happened.

Susie Murray lost two close relatives before doctors realised the whole family was at risk. The first time I telephoned her, to talk about this, she couldn’t speak to me. “I’m sorry,” she said urgently, “I’m expecting a call. My son had three peculiar episodes over the weekend. We’re trying to get hold of a consultant.” I promised to call back later, but when I did, nobody was at home. They’d rushed Alex, 11, to hospital. He was OK, but it’s hard to imagine a better insight into the life of families with Sads – constantly on alert, waiting for someone else to die.

Susie’s sister Dawn, 33, was the first in the family to die. She got up to go to the bathroom, early one morning in the autumn of 1999, and suddenly collapsed. The family was told she’d died as a result of Sads, and advised to submit to tests. But the results of those tests were misinterpreted. As a result, wrongly believing that all was well, Susie would also lose her daughter, Nadine.

Two years after Dawn died, Nadine, 21, was at home in bed. She was speaking on the telephone to her boyfriend, Matt, who was at Stansted, waiting to get on a flight. “He heard a muffled noise,” Susie recalls, “and then Nadine sighed. Matt actually heard her die, but he didn’t realise that at the time. He thought she’d been cut off.” When Nadine failed to appear at college, friends alerted the family. Susie’s husband, Alan, went round to find Nadine dead in bed, the phone at her side.

Susie and her son are now being treated, but living with the condition remains terrifying. “You have a constant, gnawing fear that it’s going to happen again. It’s Alan’s greatest fear. And imagine what it’s like for Alex. He has a deep fear that he will die in the same way.”

He also has to deal with other people. His school insisted he must not take part in sports. And it didn’t stop there. “He’s not allowed on the school bus either,” Susie says, “because nobody wants to take responsibility for him. They said, ‘It wouldn’t be nice for the other children if something happened to Alex.’”

BREAK

At the Heart Hospital, in central London, the clinic devoted to cardiac problems in the young boasts a range of sophisticated technology: electrocardiogram machines, echo scanners, halter monitors. But the clinic’s work begins with something rather less high-tech: family trees.

Constructing a pedigree for each family, explains specialist Eileen Firman, can help to show a pattern of deaths at a young age. She shows me a sample, on a sheet of A4 paper. The names have all been concealed, but the chart still provides a stirring insight into family tragedy. Individuals who have died have a line through them; the age and supposed cause of death is written underneath. It’s impossible to miss the pattern of untimely mortality. The patient’s aunt died at 39, her grandmother at 33. Two cousins died as infants, victims of cot death; two others lost their babies the same way. (It remains unclear how closely cot death is linked to Sads.)

Often patients are surprised to see the patterns. They thought their parents or grandparents died of unrelated causes, such as epilepsy, pneumonia or asthma. Others still may have been identified as drowning or dying in car accidents – while actually suffering an arrhythmia in the pool or behind the wheel.

Having completed the pedigree, families come to the clinic for tests. About half the individuals who come in are found to have a problem.

“It’s extremely difficult when one sibling has the condition and the other doesn’t,” says Firman. “There can be guilty feelings in the person that doesn’t, and often you find that it changes the dynamic of the family. Everybody devotes all their attention to the child that has it.”

As it happens, treating Sads is not horrendously complex. Prescribing betablockers can often do the job. Otherwise, patients can be fitted with an implantable cardioverter defibrillator (ICD). At a table in the Heart Hospital’s café, Firman takes an ICD out of a clear plastic bag and hands it to me. It’s covered in a dull metal, shaped like a heart – not a real heart, more like the hearts in a pack of playing cards – and weighs about the same as my mobile phone. The batteries last more than five years, Firman says. This particular model costs more than £15,000.

Once it’s been fitted, the ICD detects abnormal heart rates and, after allowing a short period for problems to rectify themselves, sends a powerful electric charge into the ventricle. It’s not pleasant – patients say it feels like being kicked in the chest – but it keeps them alive.

Firman knows of one woman whose ICD did nothing for five years. Another woman tends to get a shock every day. “She always passes out first. I was in a car with her and we were talking when suddenly her head dropped onto my shoulder. Then I felt this huge jolt go through her. She was out for about 20 seconds altogether.”

One young patient who has cheated death thanks to the ICD is James Maskell, who experienced chest pains and dizziness during exertion. After extensive testing, doctors found that his heart was stopping for several seconds during the early hours of the morning. He might have been found dead one day, like Ashley Jolly and many others. But he was lucky enough to be checked, admitted to hospital to have a device fitted, and now continues to take part in sports.

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When the government issued its first National Service Framework, describing the rights of patients, five years ago, it declined to acknowledge Sads. After all, “only” eight young people die suddenly each week, compared with nearly 2,000 people killed by coronary heart disease in the same period. But Professor William McKenna, who set up the clinic at the Heart Hospital, argues that Sads deaths are preventable, he argues, and their impact is disproportionately severe. “It’s not like losing your grandfather at 92. Families fall apart.”

The latest National Service Framework, issued last year [2005], acknowledges this. A new chapter has been written to cover diseases of the heart’s electrical systems, clearly indicating that patients should be sent to centres of excellence, no matter where they live. (NHS finance structures make GPs unwilling to refer patients outside their own area unless specifically instructed to do so.) Meanwhile, the Royal College of Pathologists is putting out advice to its members, many of whom remain unaware of Sads.

Both developments owe a great deal to charities such as Cardiac Risk in the Young, run by Alison Cox, and Sads UK, which Anne Jolly established after losing Ashley. Many families, having endured a painful bereavement, discovered the charities online, and found a valuable source of support and advice. Today, Sads UK has about 300 members. One is Penny Hurrell, whose daughter Louise died suddenly aged 12. She lost consciousness on the sofa, one evening, after a short episode of palpitations, and never came back. For the first few weeks afterwards, Penny wanted to put up Louise’s picture all over the high street, perhaps even a statue. “It was like I wanted to introduce her to everybody. The feelings of pain and loss are indescribable. And the guilt can never be appeased. I can’t believe I was not given the knowledge that would have enabled me to protect her.”

Eventually a friend gave Penny one of Anne’s leaflets. “And that opened my eyes. I didn’t feel so isolated any more. It’s tempting to think this has never happened to anyone else. When you realise that it has, you don’t feel so lonely.”

Between them, the charities have put on conferences (at one such event, when Anne stepped up to the podium to speak, she found a white feather waiting for her). They’ve recruited celebrity supporters, and set up parliamentary pressure groups. They’ve raised money to buy heart monitors, and written to 44,000 GPs across the country, explaining Sads so that – in the statistically unlikely event that doctors come across a case – they make sure to refer the entire family for checks. (Firman, at the Heart Hospital, confirms that the vast majority of families come to her through the charities, not GPs.) Sads UK wrote to Tony Blair, month after month, asking for his support. For a long period, the prime minister forwarded those letters to the Department of Health. But days after needing treatment for his own arrhythmia, in 2004, he sent back a letter of support.

Till her son died, Anne had never done anything like this. “I became a different person. I just didn’t care about approaching MPs, and medical experts. I had to do it for Ashley. I always believed I could uncover the truth.”

There’s still much to be done. Anne would like to see the introduction of a national monitoring scheme, with children attending GP surgeries when they turn 11 and recording any history of fainting and palpitations, and details of family members who may have died prematurely. The results would help to identify the children most at risk, who could then be checked more thoroughly.

She’s also encouraging schools to take advantage of the Heart Foundation’s “Heart Start” programme, in which children and teachers alike are taught resuscitation techniques. In the long term, she’d like to see that unrolled at every school, but for now she’s concentrating on schools where children from Sads families are enrolled. “You can’t do everything at once,” she says.

Her husband, who used to hate the way Anne dwelt on Ashley’s death, now says he is glad she did, because he never believed Ashley died of asthma either. Today, John Jolly is no less committed to the campaign than his wife. Their son Philip, now 21, has largely regained control over his life after all the emotional upheaval; he recently got engaged.

Because she lives locally, Penny Hurrell is able to get more closely involved with Anne’s work than other members further afield. She often comes to Anne’s house to help out: when I visit, it’s Penny who makes the tea, and hands out the biscuits. The two women are close friends. They feel like they knew each other’s children, though by definition they can never have met them.

In one of Anne’s poems, she writes about moving on: “I didn’t have a dream Ash, for you were real enough/ I have to live my life now, but I shall find this tough;/ I know what you would say, if you could speak to me/ Get on with your life, Mum, for what will be will be.”

Has she got on with her life? In many ways, she has. She hasn’t hoarded Ashley’s possessions: the few things she’s kept, such as his West Ham football kit and his old computer, are sensibly packed away. The closest to a shrine is his old website, which continues cheerfully greeting visitors who happen to come across it.

The real memorial is the ongoing campaign to raise awareness of Sads. These women are strongly motivated to prevent further avoidable death, to protect other families from what they’ve endured themselves.

It doesn’t detract from that to point out that the work is not exclusively altruistic. It answers an intensely personal need, as Penny concedes: “By doing what we are doing, I believe we’re attempting in some way to prolong our children’s lives.”

Anne, recalling that moment when her newborn son was placed in his cot beside her, agrees. “It’s my way of looking after Ashley,” she says. “But not in a sense that I would ever have imagined.”

SIDEBAR

Sudden Arrythmic Death Syndrome is actually a group of syndromes that can all be lethal. Some, such as hypertrophic cardiomyopathy, are caused by physical abnormalities that show up at post mortem. Others are related to the heart’s electrical system and can only be detected before death. Of these, the most common include Long QT, Brugada and Wolf-Parkinson-White.

For more detail, see www.sadsuk.org

SIDEBAR

Even the fittest young people die suddenly – and unnecessarily – as a result of inherited heart conditions. Daniel Yorath, son of the former Leeds United and Wales player Terry Yorath, died while playing football in the garden with his father. Ian Bell, 16, died shortly after signing for Hartlepool; John Marshall, also 16 and a junior international footballer of the year, died the day after signing for Everton. Jason Erics, a Tottenham Hotspur youth player, died aged 17. David Longhurst, 25, collapsed and died playing for York City; and Manchester City player Marc-Vivien Foe, 24, died during a live international match. Cyclists, swimmers, tennis players and cricketers have also died.

“Nearly every case I read about,” says Terry Yorath, “someone is quoted as saying: ‘He was a fit young lad.’ If someone is a fit young man they don’t think it can happen to them.”

The Premier League has recently required clubs to test young recruits, but screening for the general population is not economically viable. The best hope is to raise general awareness of the condition, and to train fitness instructors and PE teachers in resuscitation techniques.